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Menieres Disease

Menieres Disease
Chunfu Dai
Chunfu Dai
Otolaryngology Department
Otolaryngology Department
Fudan University
Fudan University

Prevalence
Prevalence

Incidence is 4/!!!!! in "apan#


Incidence is 4/!!!!! in "apan#
$/!!!!! in U%& 4'/!!!!! in
$/!!!!! in U%& 4'/!!!!! in
%(eden& !!/!!!!! in U)#
%(eden& !!/!!!!! in U)#

*he large di+erences are due to


*he large di+erences are due to
geographic& genetic& ethnic or
geographic& genetic& ethnic or
environmental factors& or di+erent
environmental factors& or di+erent
diagnostic criteria#
diagnostic criteria#

,ac-ground
,ac-ground

In .'&
In .'&

Meniere descri/ed a syndrome


Meniere descri/ed a syndrome

In 01.&
In 01.&

2allpi-e and 3ama-a(a reported the


2allpi-e and 3ama-a(a reported the
4nding of endolymphatic hydrops in
4nding of endolymphatic hydrops in
temporal /one from patients (ith MD#
temporal /one from patients (ith MD#

,ac-ground
,ac-ground

In 056&
In 056&

77OO report also de4ned t(o su/8


77OO report also de4ned t(o su/8
varieties9 cochlear and vesti/ular MD
varieties9 cochlear and vesti/ular MD

In 0.$&
In 0.$&

77O82:% updated the term MD should /e


77O82:% updated the term MD should /e
;restrictive and include only those cases
;restrictive and include only those cases
(ith complement of classic symptoms and
(ith complement of classic symptoms and
4ndings of the disease presumed to result
4ndings of the disease presumed to result
from idiopathic endolymphatic hydrops
from idiopathic endolymphatic hydrops

Pathogenesis
Pathogenesis

<adial circulation
<adial circulation

=ongitudinal >o(
=ongitudinal >o(

Pathology
Pathology

?ndolymphatic hydrops ?ndolymphatic hydrops

*he hydrops principally involves the cochlear *he hydrops principally involves the cochlear
duct and saccule /ut (ere not o/vious in the duct and saccule /ut (ere not o/vious in the
utricle and ampullae of semicirclar canals# utricle and ampullae of semicirclar canals#

*he (alls of the mem/ranous la/yrinth may *he (alls of the mem/ranous la/yrinth may
sho( areas of thinning& outpouching and sho( areas of thinning& outpouching and
ruptures ruptures

Decrease in the amount of loose connective Decrease in the amount of loose connective
tissue around the endolymphatic sac& tissue around the endolymphatic sac&
hypoplasia of vesti/ular a@ueduct& and hypoplasia of vesti/ular a@ueduct& and
decreased vasculariAation of the sac and decreased vasculariAation of the sac and
perisaccular 4/rosis (ere also o/serve# perisaccular 4/rosis (ere also o/serve#


Pathology
Pathology

Besti/ular 4/rosis Besti/ular 4/rosis

Proliferation of 4/rous tissue Proliferation of 4/rous tissue


(ithin the vesti/ule is often (ithin the vesti/ule is often
o/served (hich sometimes o/served (hich sometimes
results in the formation of /and results in the formation of /and
of 4/rous tissue /et(een the of 4/rous tissue /et(een the
under8surface of the footplate under8surface of the footplate
and the utriclar macula and the utriclar macula

*his phenomenon may account *his phenomenon may account


for a positive 2enne/erts sign& for a positive 2enne/erts sign&
(hich o/served in a/out 1!C of (hich o/served in a/out 1!C of
ears (ith MD# ears (ith MD#

Pathology
Pathology

2air cell loss


2air cell loss

In most of cases (ith MD& light Microscope has In most of cases (ith MD& light Microscope has
failed to demonstrate loss of 2C in cochlear and failed to demonstrate loss of 2C in cochlear and
vesti/ular organ# vesti/ular organ#

In advanced disease& loss of cochlear 2C& atrophy In advanced disease& loss of cochlear 2C& atrophy
of support cells& distortion and atrophy of tectorial of support cells& distortion and atrophy of tectorial
mem/rane and atrophy of cristae (ere identi4ed# mem/rane and atrophy of cristae (ere identi4ed#

?M could identi4ed fusion of cilia& disruption of ?M could identi4ed fusion of cilia& disruption of
cuticular /odies and /asal (ard displacement of cuticular /odies and /asal (ard displacement of
some O2C (ith loss of contact (ith the cuticular some O2C (ith loss of contact (ith the cuticular
plate# plate#

Pathology
Pathology

:euronal cell :euronal cell

%D: and %carpas ganglion %D: and %carpas ganglion


neuron are normal in most neuron are normal in most
patients patients

!C of temporal /ones sho(ed !C of temporal /ones sho(ed


an isolated loss of cochlear an isolated loss of cochlear
neurons in the apeE# =oss of neurons in the apeE# =oss of
nerve 4/ers in the osseous nerve 4/ers in the osseous
spiral lamina# spiral lamina#

?M identi4ed stri-ing reduction ?M identi4ed stri-ing reduction


in the num/er of a+erent nerve in the num/er of a+erent nerve
endings and a+erent synapes endings and a+erent synapes
at the /ase of /oth I2C and at the /ase of /oth I2C and
O2C# O2C#

<ecurring episode of Bertigo
<ecurring episode of Bertigo

Fhirling vertigo is usually sudden&


Fhirling vertigo is usually sudden&

Companied (ith nausea and


Companied (ith nausea and
vomiting
vomiting

Persist for several hours& attac-s last


Persist for several hours& attac-s last
longer than 64 h ma-e the diagnosis
longer than 64 h ma-e the diagnosis
of MD dou/tful
of MD dou/tful

Fluctuating 2earing loss
Fluctuating 2earing loss

?arly >uctuation of lo( ?arly >uctuation of lo(


fre@uencey hearing loss fre@uencey hearing loss
and later involvement of and later involvement of
the high fre@uencies# the high fre@uencies#

7n eventual decline over $8 7n eventual decline over $8


! years and su/se@uent ! years and su/se@uent
sta/iliAation (ith a >at sta/iliAation (ith a >at
moderate to severe hearing moderate to severe hearing
loss loss

speech discrimination speech discrimination


falling to approEimately falling to approEimately
$!C# Ma-ing the use of $!C# Ma-ing the use of
hearing aid diGcult# hearing aid diGcult#

*here is also diplacusis and *here is also diplacusis and


loudness recruitment loudness recruitment

2earing loss
2earing loss

2earing loss may /e due to the 2earing loss may /e due to the
hydrops in mem/rane of cochlea& hydrops in mem/rane of cochlea&
(hich lead to restrict movement (hich lead to restrict movement
of /asal mem/rane# of /asal mem/rane#

:eural degeneration of :eural degeneration of


unmylinated a+erent processes at unmylinated a+erent processes at
the ?M may eEplain the clinical the ?M may eEplain the clinical
4nding of progressive loss of 4nding of progressive loss of
speech discrimination despite the speech discrimination despite the
presence of intact 2C and %D:# presence of intact 2C and %D:#

,iochemical and mechanical
,iochemical and mechanical
theories
theories

Proposed /y =a(rence& McCa/e and


Proposed /y =a(rence& McCa/e and
%chu-necht that a sudden rupture of the
%chu-necht that a sudden rupture of the
thin distented mem/ranous la/yrinth
thin distented mem/ranous la/yrinth
results in >ooding of the perilymphatic
results in >ooding of the perilymphatic
space (ith potassium8rich neurotoEic
space (ith potassium8rich neurotoEic
endolymph& causing paralysis of
endolymph& causing paralysis of
sensorineuroal structure (hich in turn
sensorineuroal structure (hich in turn
results in a sudden attac- of vertigo and
results in a sudden attac- of vertigo and
hearing loss#
hearing loss#

7ural pressure and fullness
7ural pressure and fullness

One possi/ility is that


One possi/ility is that
this might /e mediated
this might /e mediated
/y sensory nerves
/y sensory nerves
supplying the middle
supplying the middle
ear aspect of the round
ear aspect of the round
and oval (indo(s#
and oval (indo(s#

7nother possi/ility is
7nother possi/ility is
sensory innervation of
sensory innervation of
dura mater surrounding
dura mater surrounding
the endolymphatic sac#
the endolymphatic sac#

:atural history of MD
:atural history of MD

%pontaneous remission of vertigo has


%pontaneous remission of vertigo has
/een reported in up to 5C of cases
/een reported in up to 5C of cases
(ith . years#
(ith . years#

%pontaneous remission ma-es assessment


%pontaneous remission ma-es assessment
of eGcacy of therapy @uite diGcult#
of eGcacy of therapy @uite diGcult#

Up to $!C of patients (ill eventually


Up to $!C of patients (ill eventually
develop symptoms from the opposite
develop symptoms from the opposite
ear# $!C of patients occurred (ithin a
ear# $!C of patients occurred (ithin a
period of 6 year
period of 6 year

:atural history of MD
:atural history of MD

=ermoyeAs syndrome9 is characteriAed /y


=ermoyeAs syndrome9 is characteriAed /y
hearing improvement that accompanies an
hearing improvement that accompanies an
acute attac- of vertigo# *he hearing remains
acute attac- of vertigo# *he hearing remains
good for a period of days or (ee-s only to
good for a period of days or (ee-s only to
gradually diminish /efore onset of the neEt
gradually diminish /efore onset of the neEt
attac-
attac-

Drop8attac-s associated (ith MD H*umar-inI9


Drop8attac-s associated (ith MD H*umar-inI9
in addition to the typical symptoms& patients
in addition to the typical symptoms& patients
eEperience a/rupt and /rief attac-s such that
eEperience a/rupt and /rief attac-s such that
they 4nd themselves thro(n to the ground
they 4nd themselves thro(n to the ground
(ith no (arning and (ith little or no vertigo
(ith no (arning and (ith little or no vertigo
after the initial fall
after the initial fall

Delayed endolymphatic
Delayed endolymphatic
hydrops
hydrops

Patients (ith pre8eEistent profound


Patients (ith pre8eEistent profound
unilateral deafness of long duration#
unilateral deafness of long duration#

%ymptoms are relieved /y


%ymptoms are relieved /y
la/yrinthectomy in the deaf ear#
la/yrinthectomy in the deaf ear#

*he original cause of deafness may


*he original cause of deafness may
have led to the hydrops#
have led to the hydrops#

Dlycerol test
Dlycerol test

2earing improvement is considered


2earing improvement is considered
suggestive of MD after oral glycerol
suggestive of MD after oral glycerol
administration# Only '!C of MD (as positive#
administration# Only '!C of MD (as positive#

2o(ever& negative test result did not eEclude


2o(ever& negative test result did not eEclude
a diagnosis& there/y& implying that the test
a diagnosis& there/y& implying that the test
(as speci4c /ut not sensitive#
(as speci4c /ut not sensitive#

In hydropic DP and ra//it ears& C7P


In hydropic DP and ra//it ears& C7P
thresholds sho( further (orse rather than an
thresholds sho( further (orse rather than an
improvement after administration of glycerol#
improvement after administration of glycerol#

Diagnosis
Diagnosis

*he diagnosis is made on the /asis of


*he diagnosis is made on the /asis of
history#
history#

Physical eEamination is usually normal


Physical eEamination is usually normal

Many tests are interesting for research


Many tests are interesting for research
purpose /ut no test proves or disproves the
purpose /ut no test proves or disproves the
diagnosis#
diagnosis#

Besti/ular& audiogram and ?:D are


Besti/ular& audiogram and ?:D are
performed to con4rm the presence of
performed to con4rm the presence of
function of the other ear#
function of the other ear#

Di+erentiate diagnosis
Di+erentiate diagnosis

,enign paroEysmal positional vertigo


,enign paroEysmal positional vertigo

Besti/ular neuritis
Besti/ular neuritis

Besti/ular drug toEicity


Besti/ular drug toEicity

=a/yrinthitis
=a/yrinthitis

2unt syndrome
2unt syndrome

Cogan syndrome
Cogan syndrome

Delayed endolymphatic hydrops


Delayed endolymphatic hydrops

Perilymph 4stula
Perilymph 4stula

,enign proEysmal position
,enign proEysmal position
vertigo
vertigo

Inappropriate eEcitation of the P%C


Inappropriate eEcitation of the P%C

=atency H84sI& transient HJminuteI


=atency H84sI& transient HJminuteI
(ith position change
(ith position change

Decreasing (ith repetitive testing


Decreasing (ith repetitive testing

MiEed vertigo torsional nystagmus


MiEed vertigo torsional nystagmus

%pontaneous resolution (ith year


%pontaneous resolution (ith year

,enign proEysmal position
,enign proEysmal position
vertigo
vertigo

Causes
Causes

2ead trauma
2ead trauma

Prolonged /edrest
Prolonged /edrest

Biral or ischemic la/yrinthitis


Biral or ischemic la/yrinthitis

Older people
Older people

Mechanism
Mechanism

dislodged otoconia trapped in P%C from


dislodged otoconia trapped in P%C from
the utricle#
the utricle#



,enign proEysmal position
,enign proEysmal position
vertigo
vertigo

Interventions
Interventions

%pontaneously resolve
%pontaneously resolve

Canalith repositioning
Canalith repositioning

7mpullary nerve section


7mpullary nerve section

,loc- posterior semicircular canal


,loc- posterior semicircular canal
Hpreventing movement of endolymph in
Hpreventing movement of endolymph in
the canalI
the canalI



Perilymph 4stula
Perilymph 4stula

*reatment
*reatment

<ecent treatments aim at control of


<ecent treatments aim at control of
vertigo#
vertigo#

Medicine Medicine

%urgery intervention %urgery intervention

:o treatment has de4nitively proven to


:o treatment has de4nitively proven to
alter the natural course of disease in
alter the natural course of disease in

Fluctuative& progressive hearing loss Fluctuative& progressive hearing loss

*innitus *innitus

7ural fullness 7ural fullness



*reatment
*reatment

%alt restriction
%alt restriction

%edative
%edative

*he -ey is to use sedative /rie>y


*he -ey is to use sedative /rie>y

Prolonged use of sedative impair


Prolonged use of sedative impair
compensation& prolongs symptoms and
compensation& prolongs symptoms and
produces a su/optimal result#
produces a su/optimal result#

Diuretic
Diuretic

*reatment
*reatment

%hort course of steroid is reasona/le %hort course of steroid is reasona/le

6 ta/s @id E5d 6 ta/s @id E5d

6 ta/s tidE6d 6 ta/s tidE6d

6 ta/s /idE6d 6 ta/s /idE6d

ta/ /idE6d ta/ /idE6d

ta/ dailyE6d ta/ dailyE6d

Intratympanic gentamicin inKectin Intratympanic gentamicin inKectin

Medical therapy fails Medical therapy fails

Intratympanic inKection is recommended Intratympanic inKection is recommended

=ess than $C patients progress to the stage =ess than $C patients progress to the stage
(here destructive treatment is indicated# (here destructive treatment is indicated#

%urgical interventions
%urgical interventions

?ndolymphatic sac
?ndolymphatic sac
shunt
shunt

Besti/ular
Besti/ular
neurectomy
neurectomy

=a/yrinthectomy
=a/yrinthectomy

?valuation of ?%%
?valuation of ?%%

Portmann 4rst proposed such


Portmann 4rst proposed such
procedures years prior to the
procedures years prior to the
description of endolymphatic hydrops#
description of endolymphatic hydrops#

De4nition of clinically signi4cant


De4nition of clinically signi4cant
hearing change H00$ 77O82:%
hearing change H00$ 77O82:%
committeeI
committeeI

a change of at least ! d, P*7


a change of at least ! d, P*7

Ford recognition score of at least $C


Ford recognition score of at least $C


?valuation of ?%%
?valuation of ?%%

7rguments on hearing outcome H(ithout


7rguments on hearing outcome H(ithout
control studyI
control studyI

2earing outcome after ?%% is no di+erent than 2earing outcome after ?%% is no di+erent than
hearing after vesti/ular nerve section& or hearing after vesti/ular nerve section& or
attri/uta/le to natural history alone# attri/uta/le to natural history alone#

%ome author indicated postoperative hearing %ome author indicated postoperative hearing
sta/iliAes or improves in $$C to .$C of patients sta/iliAes or improves in $$C to .$C of patients
after ?%% follo( up greater than 6 years# after ?%% follo( up greater than 6 years#

*innitus relief after ?%% (as reported in 685$C *innitus relief after ?%% (as reported in 685$C
of patients# of patients#

?valuation of ?%%
?valuation of ?%%

*homsens study indicated that *homsens study indicated that

su/Kective hearing loss to /e slightly signi4cantly /etter su/Kective hearing loss to /e slightly signi4cantly /etter
in the ?%% group compared (ith the place/o group# in the ?%% group compared (ith the place/o group#

O/Kective test sho(ed no signi4cant di+erence O/Kective test sho(ed no signi4cant di+erence
di+erence /et(een the t(o groups# di+erence /et(een the t(o groups#

7/ove mentioned data reeEamed /y Felling 7/ove mentioned data reeEamed /y Felling
supported supported

?%% is e+ective in management of MD and refutes the ?%% is e+ective in management of MD and refutes the
place/o e+ect place/o e+ect

*he actively shunted group have signi4cant *he actively shunted group have signi4cant
improvement in vertigo& tinnitus& and com/ined overall improvement in vertigo& tinnitus& and com/ined overall
score (hen compared (ith the place/o group# score (hen compared (ith the place/o group#

?valuation of ?%%
?valuation of ?%%

7nimal and human studies sho(ed the


7nimal and human studies sho(ed the
shunts and similar foreign materials
shunts and similar foreign materials
/ecome encapsulated /y 4/rous tissue
/ecome encapsulated /y 4/rous tissue
proliferation (hich ma-es it dou/tful that
proliferation (hich ma-es it dou/tful that
(ill remain patent#
(ill remain patent#

*he path(ay /y (hich endolymph from


*he path(ay /y (hich endolymph from
the cochea might reach the sac is /loc-ed
the cochea might reach the sac is /loc-ed
at multiple location due to collapse of the
at multiple location due to collapse of the
endolymphatic lumen#
endolymphatic lumen#


?valuation of ?%%
?valuation of ?%%

Despite the o/vious con>icting


Despite the o/vious con>icting
/eliefs concerning the /ene4t of
/eliefs concerning the /ene4t of
endolymphatic sac surgery in MD#
endolymphatic sac surgery in MD#

Most otologist routinely perform the


Most otologist routinely perform the
procedure#
procedure#

7 success rate approaching .!C& lo(


7 success rate approaching .!C& lo(
complications#
complications#

Otology group in :ashville
Otology group in :ashville

=ong8term eGcacy of ?%%


=ong8term eGcacy of ?%%

Do not o+er the patients reasona/le eEpectation Do not o+er the patients reasona/le eEpectation
for long8term& complete control of vertigo H5CI# for long8term& complete control of vertigo H5CI#

*he theoretic /ene4t of sac surgery in regard to *he theoretic /ene4t of sac surgery in regard to
the control of tinnitus& fullness& and either the control of tinnitus& fullness& and either
sta/iliAation or improvement in hearing (ere not sta/iliAation or improvement in hearing (ere not
realiAed# realiAed#

7s a result& ?%% is no longer performed#


7s a result& ?%% is no longer performed#
%u/occipital vesti/ular nerve section is
%u/occipital vesti/ular nerve section is
primarily recommendation#
primarily recommendation#

?valuation of B:%
?valuation of B:%

%urgical approaches include the middle fossa&


%urgical approaches include the middle fossa&
retrola/yrinthine and retrosigmoid
retrola/yrinthine and retrosigmoid
/su/occipital routes#
/su/occipital routes#

B:% is the gold standard for control of vertigo


B:% is the gold standard for control of vertigo
spell#
spell#

2ighly successful H0!CI in eliminating vertigo


2ighly successful H0!CI in eliminating vertigo
in patients (ith MD
in patients (ith MD

:o e+ect on hearing or other MD symptoms


:o e+ect on hearing or other MD symptoms

Complications9 facial palsy& infection&


Complications9 facial palsy& infection&
deafness#
deafness#


?valuation of B:%
?valuation of B:%

Postoperative caloric test sho(ed a


Postoperative caloric test sho(ed a
lo(er percentage of patients actally
lo(er percentage of patients actally
achieve total a/lation of vesti/ular
achieve total a/lation of vesti/ular
response#
response#

*his is due to the diGcult in severing


*his is due to the diGcult in severing
all vesti/ular nerve 4/er#
all vesti/ular nerve 4/er#

*he use of intraoperative evo-ed


*he use of intraoperative evo-ed
vesti/ular potential may overcome this
vesti/ular potential may overcome this
pro/lem#
pro/lem#

2ouse institution
2ouse institution
eEperiences
eEperiences

?%% is the 4rst line of surgical treatment


?%% is the 4rst line of surgical treatment
for MD unresponsive to medical
for MD unresponsive to medical
treatment H diuretic and vasodilator
treatment H diuretic and vasodilator
therapyI
therapyI

In case (ith disa/le vertigo unresponsive


In case (ith disa/le vertigo unresponsive
to medical treatment or failing ?%%& B:%
to medical treatment or failing ?%%& B:%
is recommended and often com/ine (ith
is recommended and often com/ine (ith
primary or revision ?%%
primary or revision ?%%

2ouse institution
2ouse institution
eEperiences
eEperiences

Concurrent ?%% and B:% does not


Concurrent ?%% and B:% does not
improvement hearing or tinnitus
improvement hearing or tinnitus
outcome over vesti/ular nerve
outcome over vesti/ular nerve
section alone
section alone

)arolins-a hospital policy
)arolins-a hospital policy

Patients (ith MD still having servicea/le


Patients (ith MD still having servicea/le
hearing (ere primarily o+ered ?=%
hearing (ere primarily o+ered ?=%

In total loss of cochlear function or persisting


In total loss of cochlear function or persisting
symptoms after a previous ?%%& intratympanic
symptoms after a previous ?%%& intratympanic
gentamicin inKection (as chosen#
gentamicin inKection (as chosen#

In patients (ith normal cochear function and


In patients (ith normal cochear function and
non Menieriform peripheral vesti/ular
non Menieriform peripheral vesti/ular
dysfunction& vesti/ular neurectomy (as
dysfunction& vesti/ular neurectomy (as
recommended#
recommended#

)arolins-a hospital policy
)arolins-a hospital policy

7s compared (ith destructive procedures&


7s compared (ith destructive procedures&
such as la/yrinthectomy and neurectomy
such as la/yrinthectomy and neurectomy
that result in severe vertigo
that result in severe vertigo
postoperatively#
postoperatively#

Intratympanic gentamicin inKection seldom


Intratympanic gentamicin inKection seldom
force the patient to re@uire /ed rest and
force the patient to re@uire /ed rest and
physical inactivity& due to slo(ly declining
physical inactivity& due to slo(ly declining
vesti/ular impairment& patients only feel
vesti/ular impairment& patients only feel
slightly unsteadness#
slightly unsteadness#

=a/yrinthectomy
=a/yrinthectomy

*ranscanal la/yrinthectomy
*ranscanal la/yrinthectomy

%emicircular canals remain intact# %emicircular canals remain intact#

Dentamicin us usually placed in the ear as (ell Dentamicin us usually placed in the ear as (ell

*ransmastoid la/ytinthectomy
*ransmastoid la/ytinthectomy

Drilling the semicircular canals Drilling the semicircular canals

Opening the vesti/ule and destroying the Opening the vesti/ule and destroying the
saccule and utricle# saccule and utricle#

Dentamicin us usually placed in the ear as (ell Dentamicin us usually placed in the ear as (ell